Collet-Sicard syndrome: an uncommon manifestation of metastatic prostate cancer.

Abstract: Metastatic spread of prostate adenocarcinoma to the temporal bone is very rare. Involvement of the jugular foramen may result in Collet-Sicard syndrome. This syndrome is characterized by paralysis of the lower four cranial nerves. A case of metastatic prostate adenocarcinoma involving the temporal bone causing Collet-Sicard syndrome is presented. This case highlights an uncommon manifestation of prostate adenocarcinoma causing symptoms referable to the occipital condyle of the temporal bone. Few cases have been reported in the literature of Collet-Sicard syndrome due to metastatic prostate cancer. This case reminds readers that awareness of atypical presentations may reduce diagnostic delay and expedite appropriate treatment.

Key Words: temporal bone metastases, prostate adenocarcinoma, Collet-Sicard syndrome, jugular foramen syndrome.

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Collet-Sicard syndrome is a rare syndrome that involves palsy of the lower four cranial nerves (CN). Trauma, vascular disease, infection and metastatic malignancies have been implicated as causes. Few cases have been reported in the literature of Collet-Sicard syndrome due to metastatic prostate cancer. This case illustrates the clinical challenge in diagnosing skull base lesions.

Case Report

A 53-year-old man reported a one week history of tongue swelling, dysarthria and difficulty swallowing solids and liquids. He had initially presented to another facility where he was diagnosed with "angioedema." He was unsuccessfully treated with steroids and HI blockers. The patient had a history of prostate cancer with bone metastasis and no allergies.

Physical examination on presentation showed that his tongue was minimally swollen, and it protruded to the right. The oral mucosa was dry, and the uvula was deviated to the left. He had dysarthria, and his voice was breathy and hoarse. The patient also had weakness turning his head to the left. The rest of his physical examination was significant for cachexia and generalized weakness.

The patient was only able to tolerate liquids. He also complained of diffuse pain in his knees and back attributed to metastatic disease.

Esophagogram showed that the barium pill got hung up in the vallecula, and many sips of water were required for the pill to be dislodged. A bone scan showed extensive metastases involving the base of the skull.

CT scan of the neck showed metastatic involvement of the cervical spine with no soft tissue abnormalities. MRI of the brain, brainstem, and cervical, thoracic and lumbar spine showed diffuse metastatic disease with involvement of the right occipital condyle (Fig. 1). Prostate specific antigen (PSA) was 391 ng/mL.

Discussion

Metastatic involvement of the jugular foramen may result in Collet-Sicard syndrome. This uncommon clinical entity was named after Frederic Collet (1870-1966) and Jean Sicard (1872-1929). Only a handful of metastatic cancers causing this syndrome have been reported. These include prostate, lung, kidney, breast, melanomas, multiple myeloma and schwannomas of the hypoglossal nerve. (1) Other noncancer causes have also been reported: head injuries (occipital condyle and first cervical vertebra fractures), internal carotid artery dissection and coiling, polyarteritis nodosa, hemangio-pericytoma, Lyme disease, carotid fibromuscular dysplasia, and idiopathic cranial polyneuropathy. (2)

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Multiple CN palsies are often diagnostic challenges because the nerves can be affected at any site along their course. CN IX-XI exit the cranial vault through the jugular foramen, whereas CN XII leaves from the hypoglossal canal close to the occipital condyle. Jugular foramen involvement may be characterized by several eponymous syndromes. Vernet syndrome describes paralysis of CN IX to XI. Collet-Sicard syndrome refers to dysfunction of the CN IX to XII, and where this is accompanied by a ipsilateral Horner syndrome, it is termed Villaret syndrome (Fig. 2). (3-5)

This patient's palsies of the lower cranial nerves were attributed to diffuse metastasis in the skull base with involvement of the right occipital condyle. Right palsy of the glossopharyngeal nerve (CN IX), vagal nerve (CN X), and hypoglossal nerve (CN XII) led to difficulty swallowing. Right sided palsy of CN X led to dysphonia (hoarseness), dysphagia, dysarthria and left uvula deviation. The patient also demonstrated difficulty turning his head to the left, which accounted for right accessory nerve (CN XI) dysfunction. Right palsy of the hypoglossal nerve (CN XII) led to deviation of the tongue to the right.

The new onset of symptoms or signs referable to the temporal bone region in patients with a known malignancy should raise the possibility of metastatic disease. In patients with prostate cancer, such spread is uncommon. In one review of the literature, of 141 cases of metastases to the temporal bone from distant primary malignancies, only 7 cases were due to prostate adenocarcinoma. It may be the initial manifestation of disseminated disease. (4) A thorough physical examination can help localize the affected region. CT can demonstrate bony erosion and soft tissue involvement. MR can show the tumor, its extent and associated perineural invasion and may reveal skull base involvement before CT findings are demonstrable.

Metastatic disease in the temporal bone associated with cranial neuropathy has an ominous prognosis. (1,4) Palliation is usually the primary focus of the clinician. Steroid therapy may be useful in reducing edema associated with the underlying malignant process and the radiotherapy which is the other mainstay of palliative treatment in this setting. Specific to prostate cancer, antiandrogen treatment may be instituted. (6,7)

Conclusions

The new onset of symptoms or signs referable to the temporal bone region in patients with a known malignancy should raise the possibility of metastatic disease. Awareness of atypical presentations may reduce diagnostic delay and expedite appropriate treatment. Although bone metastasis in prostate cancer is frequent, metastatic spread of prostate cancer to the temporal bone at the skull base affecting bulbar function has rarely been reported.

References

1. Gupta SR, Zdonczyk DE, Rubino FA. Cranial neuropathy in systemic malignancy in a VA population. Neurology 1990;40: 997-999.

2. Hsu HP, et al. A case of Collet-Sicard syndrome associated with traumatic atlas fractures and congenital basilar invagination. J Neurol Neurosurg Psychiatry 2004;75:782-784.

3. Wilson H, Johnson DH. Jugular foramen syndrome as a complication of metastatic cancer of the prostate. South Med J 1984;77:92-93.

4. Shine NP, O'Sullivan P. Collet-Sicard syndrome: a rare presentation of metastatic prostate adenocarcinoma. Auris Nasus Larynx 2005;32:315-318.

5. Schweinfurth JM, Johnson JT, Weissman J. Jugular foramen syndrome as a complication of metastatic melanoma. Am J Otolaryngol 1993;14:168-174.

6. Prashant R, Franks A. Collet-Sicard syndrome-a report and review. Lancet Oncol 2003;4:376-377.

7. Satoh H, Nishiyama T, Horiguchi A, et al. [A case of Collet-Sicard syndrome caused by skull base metastasis of prostate carcinoma] Nippon Hinyokika Gakkai Zasshi 2000;91:562-564.

Gina Chacon, MD, Irene Alexandraki, MD, and Carlos Palacio, MD

Division of Internal Medicine, Department of Medicine, University of Florida College of Medicine, Jacksonville, FL.

Reprint requests to Carlos Palacio, MD, Department of Medicine, Division of Internal Medicine, Shands-Jacksonville, 653-1 West Eight Street Jacksonville, FL 32209. Email: [e-mail]

Accepted March 30, 2006.

RELATED ARTICLE: Key Points

* Collet-Sicard is an under-appreciated syndrome.

* The new onset of symptoms or signs referable to the temporal bone region in patients with a known malignancy should raise the possibility of metastatic disease.

* Better awareness of lesions affecting the jugular foramen allows us to expedite appropriate care of patients.